Gomberg-Maitland M, Olschewski H
Dept of Cardiology, University of Chicago Hospitals, Chicago, IL, USA.
Eur Respir J. 2008 Apr;31(4):891-901. doi: 10.1183/09031936.00097107.
Prostacyclin and its analogues (prostanoids) are potent vasodilators and possess antithrombotic, antiproliferative and anti-inflammatory properties. Pulmonary hypertension (PH) is associated with vasoconstriction, thrombosis and proliferation, and the lack of endogenous prostacyclin may considerably contribute to this condition. This supports a strong rationale for prostanoid use as therapy for this disease. The first experiences of prostanoid therapy in PH patients were published in 1980. Epoprostenol, a synthetic analogue of prostacyclin, and the chemically stable analogues iloprost, beraprost and treprostinil were tested in randomised controlled trials. The biological actions are mainly mediated by activation of specific receptors of the target cells; however, new data suggest effects on additional intracellular pathways. In the USA and some European countries, intravenous infusion of epoprostenol and treprostinil, as well as subcutaneous infusion of treprostinil and inhalation of iloprost, have been approved for therapy of pulmonary arterial hypertension. Iloprost infusion and beraprost tablets have been approved in few other countries. Ongoing clinical studies investigate oral treprostinil, inhaled treprostinil and the combination of inhaled iloprost and sildenafil in pulmonary arterial hypertension. Combination of other targeted therapies with prostanoids appears to be effective and safe. After 25 yrs of continued knowledge, prostanoids remain a mainstay in the treatment of pulmonary arterial hypertension.
前列环素及其类似物（前列腺素类）是强效血管舒张剂，具有抗血栓形成、抗增殖和抗炎特性。肺动脉高压（PH）与血管收缩、血栓形成和增殖有关，内源性前列环素的缺乏可能在很大程度上导致了这种情况。这为使用前列腺素类药物治疗该疾病提供了有力的理论依据。1980年发表了前列腺素类药物治疗PH患者的首批经验。前列环素的合成类似物依前列醇以及化学性质稳定的类似物伊洛前列素、贝拉前列素和曲前列尼尔在随机对照试验中进行了测试。其生物学作用主要通过激活靶细胞的特定受体介导；然而，新数据表明其对其他细胞内途径也有影响。在美国和一些欧洲国家，依前列醇和曲前列尼尔的静脉输注以及曲前列尼尔的皮下输注和伊洛前列素的吸入已被批准用于治疗肺动脉高压。伊洛前列素输注和贝拉前列素片剂在其他少数国家已获批准。正在进行的临床研究在调查口服曲前列尼尔、吸入曲前列尼尔以及吸入伊洛前列素和西地那非联合用于肺动脉高压的情况。其他靶向治疗与前列腺素类药物联合使用似乎有效且安全。经过25年的持续认知，前列腺素类药物仍然是治疗肺动脉高压的主要药物。
